Perioperative respiratory complications caused by cystic lung malformation in Proteus syndrome

Abstract

A 7-year-old girl with Proteus syndrome, complicated with severe kyphoscoliosis and cystic lung malformation, developed respiratory complications during and after posterior spinal fusion under general anesthesia. We speculated that low oxygenation while in the prone position contributed to ventilation perfusion mismatch owing to the cystic lung malformation that existed predominantly in the right lung, and that postoperative respiratory failure was caused by initially increased bronchial mucous secretions and secondary persistent bacterial pneumonia. Cystic lung malformation rather than reduced respiratory function, although both can be a cause of mucous clearance impairment, should be considered as one of the predictive factors for perioperative respiratory failure in Proteus syndrome.

Spinal anesthesia for a rare case of Proteus syndrome

“…Therefore, it is important for anesthetists to consider specific situations. Because it is accompanied by difficult airways, skeletal system disorders, spinal canal deformities, thromboembolisms, and certain pathologies, including cystic abnormalities in the lung, emphysema, atelectasis and fibrosis, this syndrome should be further considered (5,6). Publications have particularly emphasized that difficult airways and difficult intubation may be encountered in such cases (2,7).…”

“…The most significant cause of mortality in PS is pulmonary thromboembolism induced by deep vein thrombosis caused by growing extremities (5). The likelihood of developing deep vein thrombosis is less for spinal anesthesia in comparison to general anesthesia (8).…”

Ultrasound-guided neural blockade in Proteus syndrome

“…13,14 Severe thoracic cage abnormalities could hide underlying primary disease on X-ray. 13 An argument could be made that all patients should have a preoperative chest X-ray, and if any doubt arises a high-resolution CT of the chest should be performed. Nitrous oxide should be avoided in confirmed cases of cystic lung disease.…”

“…13 Careful pulmonary interrogation and investigation are vital. Pulmonary manifestations can be classified as primary or secondary.…”

“…This is known to complicate as progressive emphysematous pulmonary disease. 13,14 Pulmonary venous varicosities can occur, and may lead to airway obstruction or rupture with life-threatening haemoptysis.…”